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临床经标准激素治疗无效应者称为激素耐药型肾病综合征,激素耐药发生机制的阐明对治疗有着重要的指导意义,近年来越来越多的研究表明基因突变、脂质代谢紊乱、免疫失调、炎症介质等因素在耐药型肾病综合征的发生机制中起着重要的作用。近年来越来越多的研究表明与基因突变有关,其中可发生NPHS2基因突变,在NPHS2基因8个外显子和部分内含子,从而导致激素耐药。GRα表达降低和GRβ表达亢进,可能是亦导致耐药。激素耐药肾病的病理类型呈多样化,表现为数种病理类型,以FSGS、MSPGN、IGM肾病为主。
Clinical standard hormone therapy ineffective who is called hormone-resistant nephrotic syndrome, hormone resistance mechanism elucidation of the treatment has an important guiding significance in recent years, more and more studies have shown that genetic mutations, lipid metabolism disorder, Immune disorders, inflammatory mediators and other factors in the pathogenesis of resistant nephrotic syndrome plays an important role. In recent years, more and more studies have shown that mutations associated with NPHS2 gene mutations may occur in NPHS2 gene exon 8 and part of intron, leading to hormone resistance. GRα expression decreased and GRβ expression hyperthyroidism may also lead to drug resistance. Hormone-resistant nephropathy pathological types were diversified, manifested as several pathological types, mainly FSGS, MSPGN, IGM nephropathy.